Difference between revisions of "Burkitt lymphoma"
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Burkitt_lymphoma,_H%26E.jpg | |||
| Width = | |||
| Caption = Starry-sky pattern. [[H&E stain]]. | |||
| Micro = medium-sized lymphoid cells of uniform size with abundant cytoplasm, interspersed macrophages | |||
| Subtypes = endemic, non-endemic, immunodeficiency associated | |||
| LMDDx = [[DLBCL]], intermediate DLBCL/BL | |||
| Stains = | |||
| IHC = [[CD20]]+, CD10+, BCL6+, [[EBER]]+, Ki-67 (100%) | |||
| EM = | |||
| Molecular = t(8;14) (q24;q32) + others | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[lymph node]] | |||
| Assdx = +/-[[EBV]] infection, +/-[[HIV]] infection | |||
| Syndromes = | |||
| Clinicalhx = very rapid growth | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = uncommon | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = | |||
| Other = | |||
| ClinDDx = | |||
}} | |||
'''Burkitt lymphoma''', abbreviated '''BL''', is an uncommon [[lymphoma]] with medium-sized cells. | '''Burkitt lymphoma''', abbreviated '''BL''', is an uncommon [[lymphoma]] with medium-sized cells. | ||
==General== | ==General== | ||
*Extremely high proliferative rate & rate of [[apoptosis]] | *Extremely high proliferative rate & rate of [[apoptosis]] | ||
*Rare in adults -- 1-2% of adult lymphomas.<ref name=omim113970>{{OMIM|113970}}</ref> | *Rare in adults -- 1-2% of adult lymphomas.<ref name=omim113970>{{OMIM|113970}}</ref> | ||
*Relatively common in children -- 30-50% of childhood lymphomas.<ref name=omim113970>{{OMIM|113970}}</ref> | *Relatively common in children -- 30-50% of childhood lymphomas.<ref name=omim113970>{{OMIM|113970}}</ref> | ||
*Although aggressive untreated, the high mitotic activity makes it relatively chemosensitive and complete remissions of 75-90% are described | |||
===Pathophysiology=== | ===Pathophysiology=== | ||
| Line 14: | Line 44: | ||
#*Found in Africa. | #*Found in Africa. | ||
#*EBV ([[Epstein-Barr virus]]) associated.<ref name=pmid12610094/> | #*EBV ([[Epstein-Barr virus]]) associated.<ref name=pmid12610094/> | ||
#Non-endemic: | #Non-endemic/sporadic: | ||
#*Typical of the BL seen in the western world; EBV negative. | #*Typical of the BL seen in the western world; EBV negative. | ||
#Immunodeficiency associated: | #Immunodeficiency associated: | ||
#*Associated with [[HIV]] infection. | #*Associated with [[HIV]] infection. | ||
#*Post-transplantation immunosuppression | |||
==Microscopic== | ==Microscopic== | ||
| Line 30: | Line 61: | ||
**Small nucleoli. | **Small nucleoli. | ||
**Relatively abundant cytoplasm. | **Relatively abundant cytoplasm. | ||
**Brisk mitotic rate. | **Brisk mitotic rate (near 100%). | ||
DDx: | DDx: | ||
*[[DLBCL]]. | *[[DLBCL]]. | ||
*B cell lymphoma unclassifiable with features intermediate between DLBCL and Burkitt lymphoma.<ref name=pmid20398809>{{Cite journal | last1 = Carbone | first1 = A. | last2 = Gloghini | first2 = A. | last3 = Aiello | first3 = A. | last4 = Testi | first4 = A. | last5 = Cabras | first5 = A. | title = B-cell lymphomas with features intermediate between distinct pathologic entities. From pathogenesis to pathology. | journal = Hum Pathol | volume = 41 | issue = 5 | pages = 621-31 | month = May | year = 2010 | doi = 10.1016/j.humpath.2009.10.027 | PMID = 20398809 }}</ref> | *B cell lymphoma unclassifiable with features intermediate between DLBCL and Burkitt lymphoma.<ref name=pmid20398809>{{Cite journal | last1 = Carbone | first1 = A. | last2 = Gloghini | first2 = A. | last3 = Aiello | first3 = A. | last4 = Testi | first4 = A. | last5 = Cabras | first5 = A. | title = B-cell lymphomas with features intermediate between distinct pathologic entities. From pathogenesis to pathology. | journal = Hum Pathol | volume = 41 | issue = 5 | pages = 621-31 | month = May | year = 2010 | doi = 10.1016/j.humpath.2009.10.027 | PMID = 20398809 }}</ref> | ||
**May be referred to as ''intermediate DLBCL/BL''.<ref name=pmid22389885>{{Cite journal | last1 = Ahn | first1 = JY. | last2 = Seo | first2 = YH. | last3 = Park | first3 = PW. | last4 = Kim | first4 = KH. | last5 = Park | first5 = MJ. | last6 = Jeong | first6 = JH. | last7 = Park | first7 = SH. | last8 = Song | first8 = YH. | title = A case of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma in a Korean child. | journal = Ann Lab Med | volume = 32 | issue = 2 | pages = 162-6 | month = Mar | year = 2012 | doi = 10.3343/alm.2012.32.2.162 | PMID = 22389885 }}</ref> | |||
===Images=== | |||
* | <gallery> | ||
Image:Burkitt_lymphoma,_H%26E.jpg | Starry-sky pattern. (WC/Uthman) | |||
</gallery> | |||
www: | |||
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1769902/figure/f1/ Burkitt lymphoma (nih.gov)]. | *[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1769902/figure/f1/ Burkitt lymphoma (nih.gov)]. | ||
| Line 49: | Line 83: | ||
==IHC== | ==IHC== | ||
Features: | Features: | ||
*CD20 +ve. | *[[CD20]] +ve. | ||
*CD10 +ve. | *CD10 +ve. | ||
*BCL6 +ve. | *BCL6 +ve. | ||
*EBER +ve. | *[[EBER]] +ve. | ||
*Ki-67 ~100%. | *Ki-67 ~100%. | ||
Others: | Others: | ||
*BCL2 -ve | *BCL2 -ve (may show weak BCL2 | ||
==Molecular pathology== | ==Molecular pathology== | ||
*t(8;14) (q24;q32) | *Isolated CMYC-IGH [[translocation]] - t(8;14) (q24;q32).<ref name=pmid12610094/> | ||
*Several other variants -- involve MYC rearrangement.<ref name=pmid12610094/><ref name=omim113970>{{OMIM|113970}}</ref> | *Several other variants -- involve MYC rearrangement with other partners.<ref name=pmid12610094/><ref name=omim113970>{{OMIM|113970}}</ref> | ||
*Should not have CCND1, BCL6 or BCL2 translocations | |||
Note: | Note: | ||
Latest revision as of 19:58, 26 December 2019
| Burkitt lymphoma | |
|---|---|
| Diagnosis in short | |
 Starry-sky pattern. H&E stain. | |
|
| |
| LM | medium-sized lymphoid cells of uniform size with abundant cytoplasm, interspersed macrophages |
| Subtypes | endemic, non-endemic, immunodeficiency associated |
| LM DDx | DLBCL, intermediate DLBCL/BL |
| IHC | CD20+, CD10+, BCL6+, EBER+, Ki-67 (100%) |
| Molecular | t(8;14) (q24;q32) + others |
| Site | lymph node |
|
| |
| Associated Dx | +/-EBV infection, +/-HIV infection |
| Clinical history | very rapid growth |
| Prevalence | uncommon |
Burkitt lymphoma, abbreviated BL, is an uncommon lymphoma with medium-sized cells.
General
- Extremely high proliferative rate & rate of apoptosis
- Rare in adults -- 1-2% of adult lymphomas.[1]
- Relatively common in children -- 30-50% of childhood lymphomas.[1]
- Although aggressive untreated, the high mitotic activity makes it relatively chemosensitive and complete remissions of 75-90% are described
Pathophysiology
- Origin cell: germinal centre B cells (favoured) vs. memory B cells.[2]
Subtypes
- Three subtypes recognized:[2]
- Endemic:
- Found in Africa.
- EBV (Epstein-Barr virus) associated.[2]
- Non-endemic/sporadic:
- Typical of the BL seen in the western world; EBV negative.
- Immunodeficiency associated:
- Associated with HIV infection.
- Post-transplantation immunosuppression
Microscopic
Features:
- "Starry-sky pattern":
- The stars in the pattern are: tingible-body macrophages.
- Tingible-body macrophages = macrophages containing apoptotic tumour cells.
- The tumour cells are the sky.
- The stars in the pattern are: tingible-body macrophages.
- Tumour cells:[2]
- Medium-sized (~1.5-2x the size of a RBC) with uniform size ("monotonous") -- key feature.
- Round nucleus.
- Small nucleoli.
- Relatively abundant cytoplasm.
- Brisk mitotic rate (near 100%).
DDx:
- DLBCL.
- B cell lymphoma unclassifiable with features intermediate between DLBCL and Burkitt lymphoma.[3]
- May be referred to as intermediate DLBCL/BL.[4]
Images
Starry-sky pattern. (WC/Uthman)
www:
Cytology
Features:
- Medium-sized lymphoid cells with small lipid vacuoles (~1-2 micrometers).[5]
Image:
IHC
Features:
Others:
- BCL2 -ve (may show weak BCL2
Molecular pathology
- Isolated CMYC-IGH translocation - t(8;14) (q24;q32).[2]
- Several other variants -- involve MYC rearrangement with other partners.[2][1]
- Should not have CCND1, BCL6 or BCL2 translocations
Note:
- MYC rearrangement is typically assessed with an ISH break apart probe.
See also
References
- ↑ 1.0 1.1 1.2 Online 'Mendelian Inheritance in Man' (OMIM) 113970
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 Bellan C, Lazzi S, De Falco G, Nyongo A, Giordano A, Leoncini L (March 2003). "Burkitt's lymphoma: new insights into molecular pathogenesis". J. Clin. Pathol. 56 (3): 188–92. PMC 1769902. PMID 12610094. http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=12610094.
- ↑ Carbone, A.; Gloghini, A.; Aiello, A.; Testi, A.; Cabras, A. (May 2010). "B-cell lymphomas with features intermediate between distinct pathologic entities. From pathogenesis to pathology.". Hum Pathol 41 (5): 621-31. doi:10.1016/j.humpath.2009.10.027. PMID 20398809.
- ↑ Ahn, JY.; Seo, YH.; Park, PW.; Kim, KH.; Park, MJ.; Jeong, JH.; Park, SH.; Song, YH. (Mar 2012). "A case of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma in a Korean child.". Ann Lab Med 32 (2): 162-6. doi:10.3343/alm.2012.32.2.162. PMID 22389885.
- ↑ Troxell, ML.; Bangs, CD.; Cherry, AM.; Natkunam, Y.; Kong, CS. (Oct 2005). "Cytologic diagnosis of Burkitt lymphoma.". Cancer 105 (5): 310-8. doi:10.1002/cncr.21307. PMID 15986398.